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Research Article: Oligocentric Castleman disease: clinical characteristics and surgical outcomes from a single-centre retrospective study

Date Published: 2025-11-19

Abstract:
Oligocentric Castleman Disease (OCD), a distinct subtype of Castleman Disease (CD) intermediate between Unicentric (UCD) and idiopathic Multicentric (iMCD) forms, remains poorly characterised. This study retrospectively analysed the clinical characteristics, treatment, and prognosis of 100 CD patients (63 UCD, 37 OligoCD). Compared with UCD, OCD patients had a higher proportion of mixed type (Mixed-CD) histology and elevated CRP/ESR levels, along with significantly poorer Progression-Free Survival (PFS) (P = 0.0067). Within the OCD cohort, debulking surgery alone or combined chemotherapy achieved an 80.0% Complete Response (CR) rate; plasmacytic type(PC-CD), non-contiguous lesions, involvement of ?3 regions, failure to achieve CR after initial treatment, and elevated baseline inflammatory markers were significant predictors of inferior PFS. Exploratory subgroup analysis divided the OCD cohort into asymptomatic and high-inflammatory groups with significantly different PFS (P = 0.042). The rate of progression to iMCD among surgically managed asymptomatic OCD patients in our study was similar to that in a large aMCD cohort predominantly managed with ‘watch-and-wait’, suggesting ‘active surveillance’ might be a more appropriate initial strategy for asymptomatic OCD. For the high-inflammatory subgroup, characterised by higher PC-CD rates and more widespread disease distribution despite effective symptom control with surgery, the post-operative relapse risk was higher. In conclusion, debulking surgery is effective for alleviating symptoms in OCD but may be unnecessary for asymptomatic patients; factors associated with a hyper-inflammatory state predict relapse, underscoring the need for careful treatment planning and exploration of novel therapeutic strategies for this high-risk subgroup.

Introduction:
Oligocentric Castleman Disease (OCD), a distinct subtype of Castleman Disease (CD) intermediate between Unicentric (UCD) and idiopathic Multicentric (iMCD) forms, remains poorly characterised.

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